Rabbit monoclonal antibody raised against synthetic peptide of human DMD.
Immunogen:
A synthetic peptide corresponding to human DMD.
Host:
Rabbit
Theoretical MW (kDa):
426.75
Reactivity:
Human
Form:
Liquid
Purification:
Affinity purification
Isotype:
IgG
Recommend Usage:
Western Blot (1:500-1:2000) The optimal working dilution should be determined by the end user.
Storage Buffer:
In PBS, 150 mM NaCl, pH 7.4 (50% glycerol, 0.02% sodium azide).
Storage Instruction:
Store at -20°C for one year. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for a longer time. Avoid repeated freezing and thawing.
Note:
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
Other Designations:
Duchenne muscular dystrophy protein,OTTHUMP00000023117,OTTHUMP00000023124,OTTHUMP00000023125,OTTHUMP00000023126,muscular dystrophy, Duchenne and Becker types